不同年龄成人语后聋患者人工耳蜗植入的疗效研究

目的　探讨不同年龄成人语后聋患者人工耳蜗植入术后的听觉言语康复效果。方法　回顾性分析44例成人语后聋患者资料,根据患者年龄分为中青年组（年龄＜60岁）、老年组（60岁≤年龄＜70岁）,高龄老年组（年龄≥70岁）。记录人工耳蜗植入前后所有患者听觉行为分级、言语可懂度分级、安静情况下言语识别率和术后12个月信噪比分别为15、10、5和0 dB噪声条件下的言语识别率。分析比较不同年龄患者手术并发症及术后疗效。结果　术后12个月,3个组患者听觉行为分级分别与同组术前比较（t 中青年组=12.90,t 老年组=9.55,t 高龄老年组=10.55,P 均＜0.01）、言语可懂度分级（t 中青年组=10.30,t 老年组=10.94,t 高龄老年组=9.98,P 均＜0.01）和安静情况下言语识别率（t 中青年组=21.71,t 老年组=21.12,t 高龄老年组=14.03,P 均＜0.01）均较术前显著提高。相同治疗阶段内,3个组间比较差异均无统计学意义（P 均＞0.05）。噪声环境下,术后12个月,信噪比为5和0 dB时,3个组言语识别率比较,差异均有统计学意义（F 5 dB=4.120,F 0 dB=5.730,P 均＜0.05）。结论　此项研究表明人工耳蜗植入可安全有效提高不同年龄段成人语后聋患者的听觉言语能力,年龄不是决定和限制人工耳蜗植入疗效的因素,但会影响噪声下言语识别能力。     

腮腺炎后双耳全聋患者人工耳蜗植入术后短期效果的观察

目的：探讨腮腺炎后双耳深度聋患者人工耳蜗植入术后短期听力与言语康复效果。方法：报告3例腮腺炎后双耳全聋患者的临床资料，观察人工耳蜗植入术后3个月时听力及言语康复效果。结果：3例均在腮腺肿胀3～7d内突然听力下降，呈极度感音神经性聋，2例伴前庭障碍症状。经保守治疗均无效。人工耳蜗植入术中均将全部电极顺利植入耳蜗鼓阶内。开机3个月时2例具有开放性言语识别能力，其中1例语言清晰准确，基本听不出聋人特有的语音特征；另1例发声准确性明显提高。余1例仅具有封闭性言语识别能力。发声准确性进步缓慢。结论：腮腺炎后双耳聋起病急，中高频听力损失最重。人工耳蜗植入术后听力康复效果好，但言语识别能力和发声的准确性与耳聋时间长短和佩戴助听器语训时间有关。     

人工耳蜗植入巡礼

人工耳蜗植入是帮助极重度感音神经性听力损失患者恢复听力和言语交流能力的可靠手段，近十年来在我国取得了长足的进步。人工耳蜗涉及到医学、听力学、生物医学工程学、教育学、心理学和社会学等诸多领域，需要多学科人员的密切配合。本文从植人手术、听力学检查与调试、听觉言语康复等方面，围绕我国近十年来人工耳蜗技术的发展做了回顾和展望，指出目前亟待解决的问题，对人工耳蜗国产化寄予期望。     

成人感音神经性聋患者人工耳蜗植入后耳鸣和抑郁状态的研究

目的:探讨成人重度、极重度耳聋患者耳蜗植入术(CI)后耳鸣和抑郁状态的变化及其相关性.方法:回顾性选择166例行CI的成人患者作为研究对象,所有患者手术前后均接受中文版耳鸣残疾评估量表(THI-C)调查和贝克抑郁量表第2版中文版(BDI-Ⅱ-C)调查,观察患者术后耳鸣改善情况、手术前后THI-C和BDI-Ⅱ-C得分情况...     

语前聋儿童人工耳蜗植入

近年来随着人工耳蜗技术的普及,越来越多重度聋的婴幼儿接受人工耳蜗植入术[1],我科于2010年开展此手术,现报道如下。 1资料与方法 1.1临床资料。3例患儿均为男性,年龄分别为1岁9个月、8岁6个月和2岁1个月。1岁左右时发现不会说话,对声响无反应,佩戴助听器行康复训练效果不理想。     

外伤引起的极重度聋行人工耳蜗植入术1例

患者，男，37岁。2002年3月因车祸头部受外伤后昏迷，在当地住院治疗，清醒后对声音无反应。后又在他院行颞骨CT检查（图1），诊断为颞骨横行骨折。双耳全聋，给予扩血管及营养神经治疗无效，配助听器后效果差。遂来我院就诊，以“双耳极重度感音神经性聋”于2003年10月22日入院。检查：双耳耳廓对称、完整，外耳道通畅，鼓膜外观正常。纯音测听示：左耳骨导0．25、0．5kHz分别为40、60dB nHL，其余频率未引出反应，气导在各个频率未引出反应；右耳骨导0．25、0．5kHz分别为40、60dB nHL，其余频率未引出反应，     

推动中国成人语后聋人工耳蜗植入

对于成人重度以上语后聋患者来说,人工耳蜗植入是重获听力的惟一有效手段,但目前仍未得到应有的重视。本文针对语后聋致病因素,成人语后聋人工耳蜗植入适应证选择,手术安全性和术后效果等方面进行阐述,旨在让更多医务工作者和从事耳聋康复事业的各界人士,对成人语后聋的人工耳蜗植入有更多的了解,从而积极推动成人语后聋人工耳蜗植入在中国的开展,让更多的耳聋患者得到及时合理的治疗,这对于提高耳聋患者生活质量具有重大的意义。     

听神经病及蜗神经发育不良儿童人工耳蜗植入的有效性

目的 通过问卷量表评估,探讨听神经病及蜗神经发育不良儿童的人工耳蜗植入效果.方法 实验组为2004年1月至2010年10月期间接受人工耳蜗植入的符合听神经病或蜗神经发育不良诊断标准、且术前至少佩戴3个月助听器并接受语言训练的患儿,共21例,其中听神经病9例,蜗神经发育不良12例.植入对照组为20例同期植入人工耳蜗的感音神经性聋患儿.所有人工耳蜗植入患儿均开机使用6个月以上.正常听力对照组为42例听力正常的儿童,按年龄分为正常听力A组(年龄在24个月以下)18例、正常听力B组(年龄24～ 48个月)16例和正常听力C组(年龄在48个月以上)8例.采用听觉行为分级(Categories of Auditory Performance,CAP)和言语可懂度分级(Speech Intelligibility Rating,SIR)两个量表进行评分,使用SPSS16.0软件对实验组得分与植入对照组和正常听力对照组进行比较.结果 听神经病组、蜗神经发育不良组、感音神经性聋组以及三组正常听力儿童的CAP得分(x±s,下同)分别为(4.44±1.50)分、(4.83±1.69)分、(4.55±1.66)分、(5.22±1.11)分、(6.75±0.45)分和(7.00±0.00)分,SIR得分分别为(2.66±1.11)分、(2.33±1.15)分,(2.40±0.75)分,(2.56±1.04)分、(4.12±0.81)分和(5.00±0.00)分.CAP和SIR得分在六组受试者间差异均具有统计学意义(x2=35.481,P＜0.001;x2 =40.549,P＜0.001);组内两两比较,听神经病组/蜗神经发育不良组与感音神经性聋组及正常听力A组得分比较,差异无统计学意义(P值均＞0.05),而与正常听力B组及C组相比,差异具有统计学意义(P值均＜0.01).结论 听神经病和蜗神经发育不良儿童接受人工耳蜗植入后,同感音神经性聋患儿一样,能够获得听觉及言语能力的进步,但落后于听力正常的同龄儿童.人工耳蜗提高此类患儿听觉及言语能力的远期效果还有待进一步随访和评估.     

脑白质异常聋儿人工耳蜗植入后效果初步分析

目的探讨对脑白质异常的重度感音神经性聋患儿,人工耳蜗植入手术的可行性及听力言语康复效果分析。方法自2012年1月至2014年12月行人工耳蜗植入术的120例(120耳)重度感音神经性聋患儿中,有脑白质异常的患儿15例。随机抽取15例脑白质无异常的聋儿作为对照组,比较两组患儿植入人工耳蜗后手术效果、开机调试结果及听力言语康复效果。结果 130例(30耳)电极全部植入耳蜗内;2所有患者术后无脑膜炎、面瘫、脑脊液漏等并发症;3所有脑白质异常患儿在人工耳蜗植入术后都有听觉;4脑白质异常儿童术后平均听阈与对照组无明显区别;5随访6至24月后,脑白质异常患儿的听觉行为分级标准(categories of auditory performance,CAP)和言语可懂度分级标准(speech intelligibility rating,SIR)与对照组比较,差异均无显著性意义(P>0.05)。结论经过严格术前评估后,人工耳蜗植入可应用于脑白质异常伴重度感音神经性聋的患儿,其术后效果与脑白质无异常的聋儿植入效果基本一致,术后的听力言语康复效果无明显差异。     

中国多道人工耳蜗植入现状

近年来，随着多道人工耳蜗技术的不断发展，有越来越多的重度感音性聋患者接受了人工耳蜗植入术，提高了生活质量，使他们能重新参与社会生活。自1995年我国引进此技术以来，截止到2003年8月已有1488例患者接受了手术。现对我国人工耳蜗植入现状、存在问题及解决方法进行探讨。     

Cochlear implantation in children with cochlear nerve deficiency

Objective

The aim of this study was to report on auditory performance after cochlear implantation in children with cochlear nerve deficiency.

Methods

A retrospective case review was performed. Five patients with pre-lingual profound sensorineural hearing loss implanted in an ear with cochlear nerve deficiency participated in the study. Postoperative auditory and speech performance was assessed using warble tone average threshold with cochlear implant, speech perception categories, and speech intelligibility ratings. All patients underwent high resolution computed tomography and magnetic resonance imaging.

Results

According to Govaerts classification, three children had a type IIb and two a type IIa cochlear nerve deficiency. Preoperatively, four patients were placed into speech perception category 1 and one into category 2. All patients had an improvement in hearing threshold with the cochlear implant. Despite this, at the last follow-up (range 18–81 months, average 45 months), only one girl benefited from cochlear implantation; she moved from speech perception category 2 to 6 and developed spoken language. Another child developed closed set speech perception and had connected speech that was unintelligible. The other 3 children showed little benefit from the cochlear implant and obtained only an improved access to environmental sounds and improved lipreading skills. None of these 4 children developed a spoken language, but they were all full-time users of their implants.

Conclusions

The outcomes of cochlear implantation in these five children with cochlear nerve deficiency are extremely variable, ranging from sporadic cases in which open set speech perception and acquisition of a spoken language are achieved, to most cases in which only an improved access to environmental sound develops. Regardless of these limited outcomes, all patients in our series use their device on a daily basis and derive benefits in everyday life. In our opinion, cochlear implantation can be a viable option in children with cochlear nerve deficiency, but careful counseling to the family on possible restricted benefit is needed.     

电诱发听性脑干反应在人工耳蜗植入中的应用

目的分析人工耳蜗植入患者耳蜗植入前电诱发听性脑干反应（electrical auditory brainstem response,EABR）的测试结果并初步探讨其意义。方法对14例不同病因、不同年龄的人工耳蜗植入患者耳蜗植入前进行测试,采用EMG外置电刺激器连接面神经探针作为刺激电极,采用Neuro Soft公司生产的Neruo Audio听觉诱发电位仪及调试软件进行EABR测试,测量并分析EABR波形,各波潜伏期和波间期,Ⅴ波波幅、阈值。结果EABR的Ⅱ、Ⅲ、Ⅳ、Ⅴ波与声诱发ABR相似,Ⅲ、Ⅴ波的潜伏期较声诱发ABR缩短,但Ⅲ~Ⅴ波间期与声诱发ABR相同。14例耳聋患者均记录到EABR的Ⅲ波/Ⅴ波,引出率为100％。4例极重度感音神经性聋未合并其他异常患者术中2.5、2.0 mA刺激Ⅲ、Ⅴ波典型,潜伏期和波间期均正常,术中神经反应遥测技术（neural response telemetru,NRT）引出,提示其听神经脑干传导通路无异常,预后效果良好。其他部分患者存在潜伏期延长或波形分化不良或刺激阈值较高等表现,提示可能由于不同因素所导致听觉传导通路异常。结论EABR是通过电刺激耳蜗来了解听觉系统对电刺激的生理反应,最接近人工耳蜗的工作状态,可以对患者听力康复的效果提供更准确的预测;特别是对于一些特殊患者,具有评估人工耳蜗植入效果的优势。而对各种特殊类型耳聋的人工耳蜗植入术前检测、术后评估数据仍需深入探索。     

重度-极重度感音性聋老年人人工耳蜗植入的初步探讨

耳聋是困扰老年人的最常见的疾病之一。目前,人工耳蜗植入(CI)是重度-极重度感音性聋且助听器效果不佳老年人的听觉和言语康复的最有效方法,但老年耳聋患者CI并不如婴幼儿和儿童听障人群开展的普及,并且老年人本身耳聋发生、听力学和听觉康复有其特殊性。从老年耳聋的发生率、耳聋常见原因和听力学特点,老年人CI的适应证,目前老年CI的开展情况,老年人CI的疗效特点等方面进行分析和讨论,并对老年耳聋患者CI的未来发展做展望。     

Advantages of cochlear implantation in prelingual deaf children before 2 years of age when compared with later implantation

OBJECTIVES: To compare the auditory abilities and speech performance of children with a profound prelingual bilateral hearing-impairment when subjected to a cochlear implant (CI) before or after 2 years of age. To analyze the complications that arose during, or as a result of, the implantation process in these groups. DESIGN: Prospective cohort single-subject, repeated-measures study of children with profound bilateral hearing impairment subjected to CI. SETTING: Tertiary referral center with a program of pediatric CI from 1991. PATIENTS: This study analyzed 130 children subjected to multichannel CI for profound prelingual bilateral hearing-impairment in two age groups: 0 to 2 (n = 36) and 2 to 6 years of age (n = 94). INTERVENTIONS: The children were evaluated before, and each year after, the intervention (for up to 5 years) with both closed-set and open-set auditory and speech perception tests. Their speech ability was evaluated according to the Peabody Picture Vocabulary and Reynell general oral expression scales. RESULTS: Auditory and speech perception tests improved significantly in all children after CI, regardless of the follow-up time. The infant's performance was better the earlier the implant was performed. Speech tests showed that the development of children treated before 2 years of age was similar to normal children, and no additional complications were observed when compared with CI in older children. CONCLUSIONS: When performed before 2 years of age, CI offers a quicker and better improvement of performance without augmenting the complications associated with such an intervention.     

Combined local anesthesia and monitored anesthesia care for cochlear implantation

IntroductionCochlear implantation has become a routine procedure for patients with hearing loss. In some patients, general anesthesia might be contraindicated due to multiple co-morbidities. We describe a successful protocol for cochlear implantation under local anesthesia with light sedation.Case reportAn 81-year-old patient presented with profound sensorineural hearing loss. Her past medical history revealed ischemic coronaropathy, managed by stenting. After multidisciplinary evaluation and clear adapted information to the patient, surgery was performed under local anesthesia with light sedation and monitored anesthesia care. The procedure lasted 70 min, and was without incident and under good conditions for the surgeon. During the intervention, the patient was comfortable. No nausea or vomiting was noted. The postoperative period was smooth and uneventful.ConclusionWe find local anesthesia with light sedation a good alternative to general anesthesia for patients where general anesthesia is contraindicated. An experienced surgical and anesthesiology team is essential to shorten the duration of the procedure.     

Double challenge: cochlear implantation in the only hearing ear with progressive hearing loss following meningitis and vestibular dysfunction after implantation

ObjectiveVestibular dysfunction associated with cochlear implantation is rare. It is usually seen in patients with otosclerosis due to spread of electrical activity throughout the demineralized bone. A 17-year old female with progressive hearing loss 2 years after meningitis and vestibular dysfunction in the implanted ear is presented in this study.FindingsThe patient had mild hearing loss in the right ear and total hearing loss on the left side because of complete ossification of the cochlea following meningitis. She had to have cochlear implantation in the right ear because of progression of hearing loss. She had successful implantation but she experienced vestibular dysfunction following activation of cochlear electrodes. Closure of two electrodes caused disruption of auditory programming. Then the patient was subjected to long term vestibular rehabilitation program.ConclusionTiming for implantation before the completion of cochlear ossification is crucial not to miss the chance for hearing restoration. However, difficulties in hearing rehabilitation due to extensive ossification can be doubled by vestibular problems triggered by stimulation of the vestibular nerve by cochlear electrodes. Attempts to reduce the balance problem will complicate auditory programming. Vestibular rehabilitation for long term helps to carry on hearing progress.     

Speech development in children after cochlear implantation

We evaluated the long-term speech intelligibility of young deaf children after cochlear implantation (CI). A prospective study on 47 consecutively implanted deaf children with up to 5 years cochlear implant use was performed. The study was conducted at a pediatric tertiary referral center for CI. All children in the study were deaf prelingually. They each receive implant before the program of auditory verbal therapy. A speech intelligibility rating scale evaluated the spontaneous speech of each child before and at frequent interval for 5 years after implantation. After cochlear implantation, the difference between the speech intelligibility, rating increased significantly each year for 3 years (P < 0.05). For the first year, the average rating remained “prerecognizable words” or “unintelligible speech”. After 2 year of implantation the children had intelligible speech if someone concentrates and lip-reads (category 3). At the 4- and 5-year interval, 71.5 and 78% of children had intelligible speech to all listeners (category 5), respectively. So, 5 years after rehabilitation mode and median of speech intelligibility rating was five. Congenital and prelingually deaf children gradually develop intelligible speech that does not plateau 5 years after implantation.     

Barriers to early pediatric cochlear implantation

Objectives

Universal newborn hearing screening has significantly improved the ability to identify patients with congenital sensorineural hearing loss (SNHL), which results in earlier treatment and better hearing and development outcomes. It is recommended that patients born with SNHL who meet criteria receive cochlear implants (CIs) by a target age of 12 months, however many children are being implanted at an older age. This study aims to describe populations of pre-lingual patients with SNHL that are at risk for delayed implantation and to identify and analyze barriers that cause this delay.

Methods

Charts of patients receiving a CI between January 2008 and June 2012 at a tertiary care cochlear implant center were reviewed retrospectively. We looked at patient demographics, age at hearing loss diagnosis, age at implantation, and etiology of hearing loss. Barriers to implantation were identified through surveys completed by team members.

Results

Fifty-seven CI recipients were identified of which 42 were in patients with pre-lingual SNHL. SNHL etiology included: cochlear dysplasia (18%), GJB2/GJB6 (17%), acquired (10%) extreme prematurity (9%), and idiopathic (46%). The median age of SNHL diagnosis for pre-lingual patients was 15 months. Compared to private insurance, public insurance status was associated with SNHL diagnosis at a significantly later median age (20.0 vs. 4.0 months, p = 0.024), and with a significantly longer median interval from diagnosis to implantation (25.5 vs. 11.0 months, p = 0.029). While cochlear implant team members identified delayed insurance approval and medical comorbidities as reasons for delayed implantation, the most significant factor identified was parental, with delayed/missed appointments or reluctance for evaluations or surgery.

Conclusion

52% of patients with pre-lingual SNHL that met criteria for CI were implanted more than 12 months after diagnosis. Having public or no insurance was significantly associated with delayed implantation. Parental barriers were most common factors cited for delays in implantation. Overcoming these delays necessitates appropriate identification of at risk patients and creating a system to educate families and chaperone them through the process.     

GJB2相关聋儿人工耳蜗术后有意义听觉整合量表评估

目的探讨GJB2基因突变致聋患儿人工耳蜗植入效果。方法对114例资料完整的2～6岁的人工耳蜗植入患者进行GJB2基因检查,根据年龄和GJB2测序结果分成2～4岁GJB2相关聋儿组和非GJB2相关聋儿组;4～6岁GJB2相关聋儿组和非GJB2相关聋儿组。术后1年,使用有意义听觉整合量表（Meaningful Auditory Integration Scale,MAIS）进行听觉康复效果问卷评估。结果114例聋儿人工耳蜗植入手术全部成功,均建立了主观听性反应。术后1年,28例2～4岁GJB2相关聋儿组的MAIS评分为25～37分,平均（34.0±2.7）分;50例2～4岁非GJB2相关聋儿组的MAIS评分为18～38分,平均（31.6±4.0）分;两组比较差异具有统计学意义（Ｐ＜0.05）。13例4～6岁GJB2相关聋儿组的MAIS评分为22～36分,平均（31.5±4.4）分;23例4～6岁非GJB2相关聋儿组的MAIS评分为25～37分,平均（31.7±3.1）分;两组比较差异无统计学意义（Ｐ＞0.05）。结论人工耳蜗患者中GJB2基因突变率高;GJB2基因突变致聋患儿的人工耳蜗植入效果稍好于非GJB2基因突变致聋患儿的人工耳蜗效果,或者他们的人工耳蜗植入效果基本一致;人工耳蜗植入可作为GJB2基因突变致聋患儿的有效治疗手段。